Craniovertebral Junction Instability in the Setting of Chiari Malformation

Described in the seminal paper by Hans Chiari in 1891, the Chiari I malformation (CMI) is a radiographic diagnosis commonly encountered by neurosurgeons and is often treated surgically with generally positive clinic outcomes. Studies have documented that 1% to 4% of patients undergoing MRI of the brain or cervical spine will be diagnosed with CMI, characterized by greater than 5 mm tonsillar herniation below the foramen magnum. More recently CMI has been described as a spectrum of disease, which includes Chiari 0, Chiari 1.5, and the complex Chiari. Primarily through multicenter clinical outcomes research, our understanding of the pathology continues to evolve.